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ANALYTICAL REPORT
Year : 2012  |  Volume : 8  |  Issue : 1  |  Page : 112-113

Classic Kaposi's sarcoma with colonic involvement: A rare presentation with successful treatment with oral etoposide


1 Department of Medical Oncology, Institute of Oncology, University of Istanbul, Istanbul, Turkey
2 Department of Pathology, Medical Faculty of Istanbul, University of Istanbul, Istanbul, Turkey
3 Department of Nuclear Medicine, Istanbul Research and Education Hospital, Istanbul, Turkey

Date of Web Publication19-Apr-2012

Correspondence Address:
Faruk Tas
Institute of Oncology, University of Istanbul, Capa, 34390, Istanbul
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.95186

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 > Abstract 

Kaposi's sarcoma (KS) is currently regarded as a low-grade vascular malignancy. KS is a multicentric and multisystem disease that involves the skin and less commonly visceral organs, such as the gastrointestinal system including colonic involvement. KS with colonic involvement in HIV-negative patients is a rare clinical manifestation. In this regard, we report an immunocompetent, HIV-negative elderly female patient with classic KS presenting with colonic involvement.

Keywords: Colonic involvement, Kaposi′s sarcoma, oral etoposide, treatment


How to cite this article:
Tas F, Keskin S, Yegen G, Gozubuyukoglu N. Classic Kaposi's sarcoma with colonic involvement: A rare presentation with successful treatment with oral etoposide. J Can Res Ther 2012;8:112-3

How to cite this URL:
Tas F, Keskin S, Yegen G, Gozubuyukoglu N. Classic Kaposi's sarcoma with colonic involvement: A rare presentation with successful treatment with oral etoposide. J Can Res Ther [serial online] 2012 [cited 2019 Nov 19];8:112-3. Available from: http://www.cancerjournal.net/text.asp?2012/8/1/112/95186


 > Introduction Top


Kaposi's sarcoma (KS) is currently regarded as a low-grade vascular malignancy. [1],[2],[3],[4] KS is a multicentric and multisystem disease that involves the skin and less commonly visceral organs, such as the gastrointestinal system including colonic involvement. KS with colonic involvement in HIV-negative patients is a rare clinical manifestation. In this regard, we report an immunocompetent, HIV-negative elderly female patient with classic KS presenting with colonic involvement.


 > Case Report Top


A 77-year-old woman patient presented to our Oncology Clinic with a one-year history of anemia, peripheral edema and skin lesions located mostly on lower limbs. On examination, plaque-like, sharply demarcated, livid bluish and partially ulcerating skin lesions were found on the lower limbs and trunk. The patients underwent biopsy in the subcutaneous soft tissue at the level of the left proximal tibia for histopathological diagnosis. A diagnosis of KS was made.

Whole body PET/CT scan study was performed and it showed diffuse colonic accumulation areas suspicious for malignancy [Figure 1]a and b. These areas were hypermetabolic on FDG-PET scanning, with a maximum standard uptake values (SUV max) of 10.8. Increased 18F-FDG accumulation in the bilateral cervical, supraclavicular, axillary, mediastinal, intra-abdominal, and right inguinal lymph nodes was also observed. Minimum and maximum standardized uptake values (SUV) for these lymph nodes were 6 and 14.7, respectively. The subcutaneous soft tissue at the level of the left proximal tibia and right femur diaphysis were accumulated by 18F-FDG with SUV max values of 2.3.
Figure 1: (a,b) Some selected slices of PET/CT scan, which demonstrate colonic FDG accumulation

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The patients underwent colonoscopy to explain involvement areas in PET-CT scan. It showed diffuse multiple segmental reddish polypoid lesions involving the ascendant and transverse regions of the large bowel. The pathological findings suggested the presence of a KS. Multiple colonoscopic biopsy specimens showed elongated spindle cells with minimal atypia [Figure 2]a and immunoreactivity for HHV8 antigen in neoplastic cells [Figure 2]b.
Figure 2: (a) Elongated spindle cells showing minimal atypia are forming slits within the lamina propria of colonic mucosa. (H and E, 20×). (b) Immunoreactivity for HHV8 antigen in neoplastic cells

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Laboratory study results were a normochromic normocytic anemia with normal white cell and platelet counts and HIV-negative.

The patient was treated with single-agent chemotherapy including oral etoposide. It was given orally 50 mg bid/day for seven days. Cycles were repeated in every 21 days. When response was assessed by PET/CT scan using standard response criteria after three cycles of chemotherapy, the colonic involvement disappeared and partial responses were seen in other involved areas.


 > Discussion Top


Classic KS primarily affects elderly males of Mediterranean, Eastern Europe, or Ashkenazi Jewish descent, as it is an uncommon malignancy in North America. [1],[2],[4] It is a slow-growing neoplasm that patients with classic KS characteristically presents as bluish-red, well demarcated, painless skin lesions in the distal extremities. [1],[2],[3],[4] These slowly grow larger and subsequently also found in more proximal regions. [2],[4] The tumors are usually asymptomatic and rarely systematically progressive. [1],[2],[4]

Despite the intestinal involvement occurs in more than half of the cases with KS who are HIV-positive and AIDS, it is uncommon in classic KS, occurs in nearly 10%, usually in the late stages of the diseases. [1],[2],[3],[4] The colonic involvement is less common than involvement of the stomach and small intestine. [4] Classic KS presenting in the gastrointestinal tract before skin involvement has rarely been reported. [4]

The isolated involvement of the colon in HIV-negative patients has been reported previously only in rare (nearly 10) cases in the literature. [2],[3],[4] Because of tumor growth primarily in the submucosa, the disease is usually asymptomatic. [1],[2],[4] However, commonly presenting clinical features include anemia from chronic blood loss, bleeding, diarrhea, protein-losing enteropathy, perforation, and obstruction. [3],[4]

The endoscopic appearance of KS lesions can range from hemorrhagic patches to discrete papules, to volcano-like lesions with central umbilication, and to large exophytic lesions projecting into the lumen. [4] However, the majority of the lesions are submucosal and are difficult to diagnose on endoscopy and, therefore, biopsy should be mandatory.

On histology, KS is a bland spindle cell lesion. [1],[2],[4] The spindle cells form vascular slits and are associated with red blood cell extravasation, hemosiderin-laden macrophages, and chronic inflammation. Intracellular or extracellular PAS-D-positive hyaline globules may be identified. The tumor cells are positive for CD31, CD34, and HHV 8 markers. [1],[2],[4] The main pathological entities in the differential diagnosis of KS involving the gastrointestinal tract include granulation tissue, angiosarcoma, and gastrointestinal stromal tumor (GIST). [4]

Descriptions of treatments of classic KS have chiefly appeared in reports of small studies at single institutions or in case reports. [1] Patients with extensive or visceral involved KS can be treated with a combination surgery, chemotherapy, and radiation or with chemotherapy. Complete remission of disseminated disease can occur after chemotherapy alone and can last for several years. Responses can be reliably obtained with various cytotoxic drugs such as vinblastine, bleomycin, liposomal anthracyclines, vinca alkaloids, and taxanes alone or in combination. Patients treated with oral etoposide had a higher rate of response than those treated with vinblastine and had less myelosuppression. [5] Likewise, we also found similar higher activity and relatively lack of toxicity in the presented patient.

In conclusion, colonic involvement of the classic KS is an uncommon presentation and its treatment with single-agent oral chemotherapy, oral etoposide, is one of the effective treatment styles.

 
 > References Top

1.Antmann K, Chang Y. Kaposi's sarcoma. N Engl J Med 2000;342:1027-38.  Back to cited text no. 1
    
2.Kahl P, Buettner R, Friedrichs N, Merkelbach-Bruse S, Wenzel J, Heukamp LC. Kaposi's sarcoma of the gastrointestinal tract: Report of two cases and review of the literature. Pathol Res Pract 2007;203:227-31.  Back to cited text no. 2
    
3.Pedulla F, Sisteron O, Chevallier P, Piche T, Saint-Paul MC, Bruneton JN. Kaposi's sarcoma confined to the colorectum: A case report. Clin Imaging 2004;28:33-5.  Back to cited text no. 3
    
4.Balachandra B, Tunitsky E, Dawood S, Hings I, Marcus VA. Classic Kaposi's sarcoma presenting first with gastrointestinal tract involvement in a HIV-negative Inuit male-A case report and review of the literature. Pathol Res Pract 2006;202:623-6.  Back to cited text no. 4
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5.Brambilla L, Labianca R, Boneschi V, Fossati S, Dallavalle G, Finzi AF, et al. Mediterranean Kaposi's sarcoma in the elderly: A randomized study of oral etoposide versus vinblastine. Cancer 1994;74:2873-8.  Back to cited text no. 5
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